Osunkwo Ifeyinwa
Children's Healthcare of Atlanta and The Department of Pediatrics Emory University, Atlanta, Georgia, USA.
Curr Opin Endocrinol Diabetes Obes. 2013 Dec;20(6):539-46. doi: 10.1097/01.med.0000436192.25846.0b.
To summarize the findings of the recent publications on sickle cell bone disease (SBD).
Individuals with sickle cell disease (SCD) are living longer and develop progressive organ damage including SBD with age. Recent studies suggest alternative radiological diagnostics such as ultrasound and scintigraphy can detect and differentiate between different forms of SBD. MRI with or without diffusion-weighted sequences remains the gold standard. Case reports of cranio-orofacial SBD highlight the rarity of this presentation. Vitamin D deficiency is highly prevalent at all ages, but may not be an independent risk factor for avascular necrosis (AVN). Gene polymorphisms of the Annexin A gene may predict AVN in SCD. A recent study demonstrated reduced days with pain and improved physical activity quality of life following high-dose vitamin D therapy. The high rates of osteopenia and osteoporosis in SCD support the need for research addressing this rising public health problem. Lastly, results of total hip arthroplasty for AVN in SCD has improved significantly over time with the use of cementless prosthetic material and improved supportive care.
SBD remains poorly studied. Prospective randomized studies targeting predictors, diagnostics, prevention, and treatment options for SBD are sorely needed.
总结近期关于镰状细胞骨病（SBD）的出版物的研究结果。
镰状细胞病（SCD）患者的寿命延长，且随着年龄增长会出现包括SBD在内的进行性器官损害。近期研究表明，超声和闪烁扫描等替代性放射学诊断方法可检测并区分不同形式的SBD。有无扩散加权序列的MRI仍是金标准。颅颌面SBD的病例报告突出了这种表现的罕见性。维生素D缺乏在各年龄段都非常普遍，但可能不是无血管性坏死（AVN）的独立危险因素。膜联蛋白A基因的基因多态性可能预测SCD中的AVN。最近一项研究表明，大剂量维生素D治疗后疼痛天数减少，身体活动生活质量得到改善。SCD中骨质减少和骨质疏松的高发生率支持了针对这一日益严重的公共卫生问题进行研究的必要性。最后，随着非骨水泥假体材料的使用和支持性护理的改善，SCD中AVN的全髋关节置换术结果随时间推移有了显著改善。
SBD的研究仍然不足。迫切需要针对SBD的预测因素、诊断方法、预防措施和治疗选择进行前瞻性随机研究。
